Pulmonary Manifestations of Systemic Disease: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II |

Rituximab for Diffusealveolar Hemorrhage in Granulomatosis With Polyangiitis: A Case Report FREE TO VIEW

Biswajit Mishra
Author and Funding Information

Fortis Hospital, Bhubaneswar, India

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1101A. doi:10.1016/j.chest.2016.08.1208
Text Size: A A A
Published online

SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Diffuse Alveolar Haemorrhage(DAH) is a rare manifestation of Granulomatosis with Polyangiitis (GPA) and is associated with high mortality.Cyclophosphamide, steroids along with plasmapheresis have been used for the treatment, however a proportion of patients may not optimally respond to these modalities.Rituximab(RTX) has been shown to be effective in GPA but evidence for use in DAH is lacking.

CASE PRESENTATION: A 68 yr old male who was diagnosed to have GPA (BVAS-15) two months back was treated with intravenous Methylprednisolone(IVMP),IV cyclophosphamide and later switched on to oral steroids.He was clinically stable till he presented to us with acute shortness of breath.He was tachycardic, tachypneic and hypoxic.Investigations revealed low hemoglobin,leucocytosis and raised inflammatory markers(ESR-120mm/hr,CRP- 60 mg/l).Infection screen and pro-calcitonin were negative.Urine routine analysis showed proteinuria 2+ and RBC casts. Chest X-Ray showed extensive confluent opacities bilateral lung fields.Patient was intubated and bronchoscopy done revealed blood in the airways,lavage showed hemosiderin-laden macrophages.A diagnosis of DAH secondary to GPA was made (BVAS-27).IVsteroids and plasmapheresis(six cycles)given while continuing IV cyclophosphamide.However,no improvement in clinical status and continued to be on ventilator(FiO2>50%).In the meantime,he developed new digital gangrene in left index finger. Inspite of 5gm IVMP and six cycles of plasmapheresis,he didnot improve. RTX 1gm two doses two weeks apart was given.After one week of RTXsignificant improvement with resolution of chest symptoms and x ray findings,improvement in ventilator parameters by day10.Cyclophosphamide was stopped,azathioprine initiated and tapering of oral steroids continued.In last one year,he has remained in complete remission till date(BVAS- 3).

DISCUSSION: DAH occurs as a consequence of pulmonary capillaritis in ANCA-associated vasculitides(AAV) with an incidence of 7-45% in GPA.Mortality associated with DAH is approximately 60%.Haemoptysis and dyspnoea are the common clinical presentations. Cyclophosphamide and steroids have been the mainstay of treatment for long and upto 10% of patients remain refractory to these drugs1.RTX has recently been shown to be effective both for induction and maintenance of remission with a good safety profile,however, data regarding the efficacy in DAH is limited.Since our patient did not show optimal improvement despite steroids, cyclophosphamide and plasmapheresis,RTX was our next available option.

CONCLUSIONS: RTX could be a useful option for the nearly-fatal DAH but numbers reported from literature are small.Data from larger randomized trials are required to prove its usefulness as an add-on therapy or as a 1st option for DAH in AAV.

Reference #1: Jayne D,Rasmussen N,Andrassy K et al.(2003)A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies.N Eng J Med349,36-44.

DISCLOSURE: The following authors have nothing to disclose: Biswajit Mishra

No Product/Research Disclosure Information




Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543